Pieterman Carolina R C, Grozinsky-Glasberg Simona, O'Toole Dermot, Howe James R, Ambrosini Valentina, Belli Susana H, Andreassen Mikkel, Begum Nehara, Denecke Timm, Faggiano Antongiulio, Falconi Massimo, Grey Jo, Knigge Ulrich P, Kolarova Teodora, Niederle Bruno, Nieveen van Dijkum Els, Partelli Stefano, Pascher Andreas, Rindi Guido, Ruszniewski Philippe, Stättner Stefan, Vandamme Timon, Valle Juan W, Vullierme Marie-Pierre, Welin Staffan, Perren Aurel, Bartsch Detlef K, Kaltsas Gregory K, Valk Gerlof D
Department of Endocrine Oncology, University Medical Center Utrecht, Utrecht, the Netherlands.
Neuroendocrine Unit, ENETS Center of Excellence, Division of Medicine, Hadassah Medical Center and Faculty of Medicine, The Hebrew University, Jerusalem, Israel.
J Neuroendocrinol. 2025 Jan;37(1):e13468. doi: 10.1111/jne.13468. Epub 2024 Nov 26.
Multiple Endocrine Neoplasia type 1 (MEN1) Clinical Practice Guidelines (2012) are predominantly based on expert opinion due to limited available evidence at the time, leaving room for interpretation and variation in practices. Evidence on the natural course of MEN1-related neuroendocrine tumours (NET) and the value of screening programs has increased and new imaging techniques have emerged. The aim of this study is to provide insight in the current practices of screening and surveillance for MEN1-related NETs in ENETS Centers of Excellence (CoEs). A clinical practice questionnaire was distributed among all 65 ENETS CoEs. Response rate was 91% (59/65). In 14% of CoEs <10 patients, in 50% 10-49, in 31% 50-100 and in 3 centres (5%) >100 patients with MEN1 are seen. Practices with regard to screening and surveillance of NETs were markedly heterogeneous. Differences between countries were noted in the use of gut hormones for biochemical screening and the choice for imaging modality for screening/surveillance of pancreatic NETs (PanNETs). Magnetic resonance imaging (MRI) is the preferred modality for screening and surveillance of PanNETs, whereas this is computed tomography (CT) for thoracic NETs. Practices regarding screening for thoracic NETs were more homogeneous among larger volume CoEs, with longer screening intervals. The majority of CoEs tailored the surveillance of small pancreatic and lung NETs to observed growth rate. 68% of CoEs advise patients with clinical MEN1 with negative genetic testing to undergo periodic screening like mutation-positive patients. In conclusion, there is still marked heterogeneity in practice, although there are also common trends. Differences were sometimes associated with volume or country, but often no association was found. This underscores the need for clear and evidence-based practice recommendations.
1型多发性内分泌肿瘤（MEN1）临床实践指南（2012年）主要基于专家意见，因为当时可用证据有限，这使得实践中存在解释和差异的空间。关于MEN1相关神经内分泌肿瘤（NET）自然病程及筛查项目价值的证据有所增加，且出现了新的成像技术。本研究旨在深入了解欧洲神经内分泌肿瘤学会卓越中心（CoE）对MEN1相关NETs的筛查和监测现状。向所有65个欧洲神经内分泌肿瘤学会卓越中心发放了一份临床实践调查问卷。回复率为91%（59/65）。在14%的卓越中心，每年诊治的MEN1患者少于10例；50%的卓越中心为10 - 49例；31%的卓越中心为50 - 100例；3个卓越中心（5%）每年诊治的MEN1患者超过100例。NETs的筛查和监测实践存在显著异质性。在用于生化筛查的肠道激素使用以及胰腺NETs（PanNETs）筛查/监测的成像方式选择上，各国存在差异。磁共振成像（MRI）是PanNETs筛查和监测的首选方式，而胸部NETs则首选计算机断层扫描（CT）。在病例量较大的卓越中心，胸部NETs的筛查实践更为统一，筛查间隔更长。大多数卓越中心根据观察到的生长速度对小的胰腺和肺NETs进行监测。68%的卓越中心建议临床诊断为MEN1但基因检测阴性的患者像基因检测阳性的患者一样接受定期筛查。总之，尽管存在一些共同趋势，但实践中仍存在显著异质性。差异有时与病例量或国家有关，但通常未发现关联。这凸显了制定明确且基于证据的实践建议的必要性。
